Article
Human iPSC-derived astrocytes generated from donors with globoid cell leukodystrophy display phenotypes associated with disease.
PloS one - 1 Jan 2022
Lieberman Richard, Cortes Leslie K, Gao Grace, Park Hyejung, Wang Bing, Jones Patrick L, Hunter R Bridge, Leonard John P, Barker Robert H
Abstract excerpt
Globoid cell leukodystrophy (Krabbe disease) is a fatal neurodegenerative, demyelinating disease caused by dysfunctional activity of galactosylceramidase (GALC), leading to the accumulation of glycosphingolipids including psychosine. While oligodendrocytes have been extensively studied due to their high levels of GALC, the contribution of astrocytes to disease pathogenesis remains to be fully elucidated. In the...
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